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Review the diagnostic pathway for pulmonary arterial hypertension (PAH) in congenital heart disease (CHD).
Understand and interpret right heart catheterisation data in pulmonary hypertension related to CHD.
Recognise the typical presentation of PAH in patients with CHD.
Explore the treatment options for PAH-CHD.
Review and understand the management of common emergencies in PAH-CHD.
Pulmonary hypertension (PH) is defined as a mean pulmonary arterial pressure (PAP) above 20 mm Hg, determined by right heart catheterisation. PH is classified into five distinct groups based on underlying pathophysiology and haemodynamics (figure 1).1 2 Patients with pulmonary arterial hypertension (PAH) have precapillary PH haemodynamics, defined as a mean PAP above 20 mm Hg, a pulmonary vascular resistance (PVR) >2 Wood units (WU) and a pulmonary capillary wedge pressure (PCWP) ≤15 mm Hg.2 PAH encompasses conditions with similar pathophysiological characteristics of the pulmonary circulation, and includes PAH associated with congenital heart disease (PAH-CHD), which is the focus of this article (figure 2) 2. PAH affects approximately 10% of patients with CHD, and PAH-CHD accounts for almost a third of the adult PAH population.3 Delays in the diagnosis of PAH can be avoided if patients with CHD are regularly monitored in specialist tertiary centres, where clinicians are vigilant to all complications of CHD.2 4 5
PF and KK are joint first authors.
PF and KK contributed equally.
Contributors KK and PF were responsible for manuscript writing and prepared the manuscript with contributions from all coauthors.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Commissioned; internally peer reviewed.
Author note References which include a * are considered to be key references.
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